Children's Doctor

Dermatology - Spring 2012

Getting to the Bottom of Things

Melinda V. Jen, MD, and Albert C. Yan, MD

rash on pelvic areaFigure 1. RashA 4-month-old historically healthy boy developed symptoms of cough and rhinorrhea. After 2 weeks, these symptoms began to subside, but he then began having daily fevers around 101°F for about a week. A diaper rash appeared that did not respond to daily use of topical zinc oxide paste. His parents thought this diaper rash was making him irritable, so his parents brought him to the Emergency Department.

Discussion: The diagnosis is perineal desquamative rash of Kawasaki disease (KD).

Classic KD is a systemic inflammatory disorder characterized by medium-vessel vasculitis. Clinically, patients typically present with ≥5 days of fever and 4 of 5 of the following: changes in extremities, rash, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, and cervical lymphadenopathy. The polymorphous rash appears several days after the start of the fevers. Although the rash can appear scarlatiniform, morbillliform, urticarial, or erythema multiforme-like, a prominent feature is accentuation in the skin folds.

Classic Clinical Criteria for Kawasaki disease

In this patient, an erythematous, desquamating perineal rash is described. This presentation represents an under-recognized finding in KD. In 1 case series, 67% of those fulfilling criteria for KD demonstrated a perineal desquamative eruption during the first week of onset of symptoms. Perineal erythema and desquamation should raise the suspicion of KD, particularly in the appropriate clinical context. In addition to the classic clinical criteria, there are other clinical findings that support the diagnosis of KD. These include arthritis, urethritis, and irritability, a possible manifestation of aseptic meningitis. The major sequela of KD is its cardiac complications, most commonly coronary artery disease, but also myocarditis, decreased ventricular contractility, and valvular disease. In untreated patients, 15% to 25% of patients develop coronary artery abnormalities.

Other Clinical Findings for Kawasaki disease

Cardiovascular findings
  • Congestive heart failure, myocarditis, pericarditis, valvular regurgitation
  • Coronary artery abnormalities
  • Aneurysms of medium-sized noncoronary arteries
  • Raynaud's phenomenon
  • Peripheral gangrene
Musculoskeletal findings
  • Arthritis
  • Arthralgia
Genitourinary findings
  • Urethritis/meatitis
Gastrointestinal findings
  • Diarrhea, vomiting, abdominal pain
  • Hepatic dysfunction
  • Hydrops of gallbladder
Central nervous system findings
  • Extreme irritability
  • Aseptic meningitis
  • Sensorineural hearing loss
Other findings
  • Erythema, induration at BCG inoculation site
  • Anterior uveitis
  • Desquamating groin rash
 

Irritant contact dermatitis manifests with well-demarcated erythema and maceration, and typically spares the intertriginous folds. Superinfection of pre-existing irritant contact dermatitis by yeast organisms may occur, a setting in which intertriginous areas may become involved, and satellite papules and pustules appear. Allergic contact dermatitis in the diaper area may develop as more geometrically distributed rashes. Look for patterned rashes that follow the elastic bands (rubber-related allergens) and the colored areas of the diaper (disperse-dye allergens). Recently, reports of allergic contact dermatitis to the material in Pampers Drymax™ have been described although the specific allergen is yet to be determined. Known diaper-related allergens that may be involved include mercaptobenzothiazole, fragrances, cyclohexylthiophtalimide, p-tertbutylphenol formaldehyde resin, and sobritan sesquioleate. Reactions may also occur in response to diaper wipes which may also contain fragrances or other sensitizing chemicals.

Perianal streptococcal disease classically affects the perianal areas, although intertriginous involvement has been described. Affected patients usually manifest with bright, fire-engine-red color, tenderness, and a characteristic foul odor associated with these areas.

The entity most difficult to distinguish from the perineal rash of KD is recurrent perineal toxic erythema (toxin-mediated perineal erythema).

Perineal Desquamative Rashes
Condition Clinical Findings Key Features
Kawasaki disease Perineal erythema and desquamation Fever; polymorphous exanthem; lymphadenopathy; mucous
membrane findings; extremity changes; coronary artery aneurysms
Irritant contact dermatitis Well-demarcated erythema and maceration Sparing of the intetriginous areas; lack of systemic findings
Allergic contact dermatitis Well-demarcated geometric erythema,
maceration
Often follows distribution of allergen in the diaper; look for patterns associated with elastics or diaper dyes
Candidal diaper dermatitis Erythema, maceration, satellitosis Satellite lesions and involvement of intertriginous areas is prominent;
Lack of systemic findings
Perineal streptococcal disease Painful or tender fiery red erythema in
intertriginous and perianal distribution
Foul odor is commonly associated
Toxin-mediated perineal
erythema
Perineal erythema and desquamation Prodromal pharyngitis; afebrile or fevers of short duration; cultures of
throat positive for Staphylococcus aureus or group A beta-hemolytic
Streptococcus; responsive to antibiotic therapy; recurrent phenomenon

This phenomenon presents as asymptomatic areas of desquamative erythema following pharyngitis. Affected patients may also show erythema and swelling of the hands and feet as well as a strawberry tongue. In contrast to KD, however, patients may be afebrile or have generally short-lived fevers, the patients show positive throat cultures, and they respond promptly to appropriate antibiotic therapy. Moreover, patients with this phenomenon often go on to have recurrences. This condition presumably results from a superantigen-related response to toxin-producing Staphylococcus aureus or group A beta-hemolytic Streptococcus. Other rare but important causes of atypical diaper dermatitis include those associated with zinc deficiency, Langerhans cell histiocytosis, and glucagonoma syndrome.

References and Suggested Readings

Friter BS, Lucky AW. The perineal eruption of Kawasaki syndrome. Arch Dermatol. 1988;124(12):1805-1810.

Newburger J, Fulton D. Kawasaki disease. Curr Opin Pediatr. 2004;16(5):508-514.

Manders SM. Toxin-mediated streptococcal and staphylococcal disease. J Am Acad Dermatol.1998;39:383-398.

Jacob SE, et al. Allergic contact dermatitis to Pampers™ Drymax. Pediatric Dermatology. 2012 February 3. [Epub ahead of print.]

Honig PJ, et al. Streptococcal intertrigo: an underrecognized condition in children. Pediatrics. 2003;112(6):1427-1429.

Manders SM, et al. Recurrent toxin-mediated perineal erythema. Arch Dermatol. 1996;132:57-60.

Patrizi A, et al. Recurrent toxin-mediated perineal erythema: eleven pediatric cases. Arch Dermatol. 2008;144(2):239-243.

Exuberant Enterovirus Infections

Leslie Castelo-Soccio, MD, PhD, James Treat, MD, and Albert C. Yan, MD

An 11-month-old male presents to the Emergency Department (ED) with extensive 1 to 4 mm vesicles on his hands, feet, oral mucosa, buttocks, arms, and legs. He had been febrile to 102° to 104° F for 2 days prior to developing sores in his mouth and blisters on his handsvesicles on handsFigure 2. Vesicles on the hands of an infant with enterovirus infection and feet. In the ED, he was febrile to 102° F. Parents note that the vesicles initially started on the hands and mouth, but in the last few days have spread to his feet, arms, legs, buttocks, and face. He is eating less than usual. He has no upper respiratory symptoms. He has a history of mild atopic dermatitis. He is up to date on his vaccinations and attends daycare. A swab was taken from the base of the vesicles for PCR to test for enterovirus as well as herpes simplex virus and varicella and is consistent with enteroviral infetction.

exuberant presentation of vesicles on the posterior legsFigure 3. Exuberant presentation of vesicles on the posterior legs of an infant with enterovirus infectionDiscussion: Hand, foot, and mouth disease (HFMD) is a common generally benign febrile viral disease of infants and children under 5 years of age. It can have exuberant presentations. This year an increased number of cases combined with increased severity of blistering on hands and feet as well as more generalized blistering presentations have been noted in the Philadelphia area. Reports from Asia also note an increased number and severity of infection in China and Vietnam. Older children and adults are occasionally affected, but tend to develop milder form of illness compared to younger children. Coxsackie virus A16 and enterovirus 71 (EV71) are the most common causes, although many other enteroviruses are also associated with HFMD including Coxsackie virus A4, A5, A6, A7, A9, A10, A24, B2 to B5, and echovirus 18. Infection is spread by direct contact with the virus. Virus is shed from the nose, saliva, blister fluid, and stool. The virus can remain in the body for weeks even after the patient has recovered completely. Infection results in immunity to the specific virus that caused HFMD. It is rare for an immunocompetent child to develop HFMD again.

enteroviral infectionFigure 4. Extremity of 4-year-old with enteroviral infection
hand with rashFigure 5. Hand foot and mouth disease in child more than 10 years of age
 

In its most exuberant forms it is often mistaken for variciella or eczema herpeticum. To help differentiate these entities, it is important to remember that varicella tends to start on the central abdomen and move outward. vesicles on handsFigure 6. Crusted vesicles around mouth in a 3-year-old patient with enterovirus infectionIt is usually present with multiple stages of vesicles and crusting. Eczema herpeticum is usually more clustered and uniform in size with deeper resultant erosions. Eczema herpeticum should be located in areas of prior atopic dermatitis and in general spares the perianal area whereas hand, foot and mouth (and buttocks) disease does not. HFMD tends to be more widespread in frictional areas.

In general, the disease is mild and self-limited except in the case of EV71 infections, which may incur serious complications. Complications include poliomyelitis-like acute flaccid paralysis, brainstem encephalitis, and other severe systemic disorders, including pulmonary edema and cardiorespiratory collapse. Clinical predictors of severe disease include high temperature and lethargy. A lumbar puncture might reveal pleocytosis. Many diagnostic tests are available, but PCR of throat swabs and swabs from the base of the vesicle, if available, is among the most efficient. Results are usually available within 24 hours.

In general, care is supportive. It is important to keep children hydrated since some may get dehydrated if they are not able to swallow enough liquids because of painful mouth sores. Antipyretics can be helpful to reduce pain and fever. Mouthwashes that numb mouth pain can be helpful in older children. Children with severe mouth erosions occasionally need admission for IV hydration. Bland emollients like petrolatum for the eroded areas of the hands, feet, and body can be helpful.

Washing hands often with soap and water, especially after changing diapers and using the toilet is critical. Disinfecting dirty surfaces including toys is recommended by the Centers for Disease Control, which advocates mixing 1 tablespoon of bleach to 4 cups of water. Avoiding close contact, including kissing and hugging, or sharing utensils and cups with people with HFMD is recommended.

References and Suggested Readings

Center for Disease Control Website. http://www.cdc.gov/hand-foot-mouth/

Osterback R, Vuorinen T, Linna M, Susi P, Hyypiä T, Waris M. Coxsackievirus A6 and hand, foot, and mouth disease, Finland. Emerg Infect Dis. 2009;15:1485-1488.

Shieh WJ, Jung SM, Hsueh C, Kuo TT, Mounts A, Parashar U, et al. Pathologic studies of fatal cases in outbreak of hand, foot, and mouth disease, Taiwan. Emerg Infect Dis. 2001;7:146-148.

Wong, SS, Yip, CC, Lau, Sk, Yuen, KY. Human enterovirus 71 and hand, foot and mouth disease. Epidemiol Infect. 2010;138(8):1071-1089.

Xu, W, Liu, CF, Yan, L, et al. Distribution of enteroviruses in hospitalized children with hand, foot and mouth disease and relationship between pathogens and nervous system complications. Virol J. 2012; 9;9(1):8.
 

Sitting in the Hot Seat: Raising Awareness of Infant Car Seat Dermatitis

Patrick J. McMahon, MD

vesicles on armsFigure 7. Upper armsAn 11-month-old male presented to the CHOP Emergency Department in August 2011 with a 1-month history of widespread pruritic, crusted, weeping plaques. Two weeks prior to presentation, he was admitted to a nearby hospital for “superinfected eczema” and improved after systemic antibiotics and corticosteroids. However, his rash and intense pruritus recurred within 7 days after discharge. Upon close examination, his weeping plaques were symmetrically distributed over posterior legsFigure 8. Posterior legsthe extensor upper armsand posterior legs. He also had scattered pink papules and ill-defined patches on his posterior trunk. These anatomic locations coincided with the areas of his skin that were in direct contact with his car seat. He was diagnosed with contact dermatitis, which was felt to be most consistent with car seat dermatitis, along with a concurrent “id reaction.”

Discussion: As the warmer months approach here in Philadelphia and infants begin to don short-sleeved onesies, it is important to keep “car seat dermatitis” in mind as a newly recognized form of contact dermatitis. In spring 2011, this entity was reported in a case series of 21 patients, aged 3 to 14 months, seen in a private practice pediatric dermatology clinic in Texas. Apart from this report, little else is available in the medical literature on this subject. However, online parenting blogs dating back to 2008 have testimonials from clever parents who had noticed an itchy rash on their children that was worse in areas on skin that was in direct contact with the car seat. This led them to ask, “Is it possible to be allergic to a car seat?” This reaction was found to be more commonly associated with car seats lined with a shiny, nylon-like material and was more prevalent in warmer months. The areas of involvement included the posterior legs (100%), elbows (95%), and the occipital scalp (43%). As in our patient, 2 of the cases in the reported case series had an “id reaction,” a more widespread eczematous papular eruption distant from the site exposure also known as autosensitization dermatitis.

The authors were unable to determine the exact causative agent in the car seat lining despite contacting the manufacturers. Ingredients that are being investigated include a flame retardant, a preservative used as a mold inhibitor known as dimethylfumurate, or another yet unknown agent. Given that many cases are associated with excessive sweating, some experts have theorized that this reaction could represent an irritant rather than allergic contact dermatitis. It has been suggested that epicutaneous patch testing using different portions of the fabric lining found in these particular car seats may help confirm this diagnosis and determine exactly which component of the car seat is to blame.

Once this diagnosis is suspected, patients benefit from replacement of the car seat along with topical steroids. As heat, sweat, and short summer clothing are felt to play a role in facilitating this contact dermatitis, keeping cool and applying a barrier between the child and the car seat lining could be helpful in cases where buying a new car seat is not feasible. Upon follow up, our patient had improved markedly after treatment with mid-potency topical steroids and purchase of a new car seat with a different lining.

References and Suggested Readings

Ghali FE. “Car seat dermatitis:” a newly described form of contact dermatitis. Pediatr Dermatol. 2011 May-Jun;28(3):321-6.

Is it possible to be allergic to a carseat? UPDATE — yes, it is. Mothering. Website. www.mothering.com/community/t/893494/is-it-possible-to-be-allergic-to-a-carseat-update-yes-it-is. Accessed March 26, 2012.

Pfeiffer A. Clinical Rounds Blog: Car Seats Linked to Dermatitis. Pediatric News. Website. http://www.pediatricnews.com/index.php?id=7791&cHash=071010&tx_ttnews%5btt_news%5d=95480. Accessed March 26, 2012.

Chang M. Car Seat Contact Dermatitis. Journal Watch Dermatology. September 23, 2011. Website. http://dermatology.jwatch.org/cgi/content/full/2011/923/1. Accessed March 27, 2012 (free registration required).

Referral Information

To refer a patient to CHOP’s Division of Dermatology, please call 215-590-2169 and press “1” at the prompt.

For expedited appointment requests, please fill out the form on www.chop.edu (search for “Dermatology Expedited Appointment”) and fax the completed form to 215-590-4948. Find more information, including the CHOP Care Network Specialty Care Center locations that offer Dermatology appointments, at www.chop.edu/dermatology.
 

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